98%
921
2 minutes
20
We describe an elderly patient presenting with pneumothorax, cystic lung disease and a scalp lesion. The pneumothorax resolved after placing a chest tube and suction but recurred within a week. Progression of cystic features was also seen, and biopsies of the lung and scalp lesions were performed. Immunohistochemistry was positive for markers of endothelial cells (CD31 and ERG) and negative for markers expected to be positive in alveolar cells (keratin AE1/AE3 and TTF-1), supporting the diagnosis of metastatic angiosarcoma. Palliative chemotherapy did not prevent progression and the patient expired soon after. In describing the clinico-radiological correlation of metastatic angiosarcoma, we also briefly describe the approach to cystic lung disease. Understanding the pathophysiology of cyst formation in metastatic angiosarcoma may help clinicians to better appreciate and manage the full spectrum of cystic lung disease, especially with atypical features.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1136/bcr-2023-258333 | DOI Listing |
J Cyst Fibros
September 2025
Division of Paediatric Pulmonology; University of Cape Town, South Africa; Red Cross War Memorial Children's Hospital, Cape Town, South Africa.
Background: Cystic fibrosis (CF) is a genetic disorder that remains underrecognized across Africa, where limited diagnostic capacity, low awareness, and competing health priorities contribute to delayed or missed diagnoses [1-4]. Although increasing data suggests CF is more prevalent than previously believed in Africa, survival remains poor [1]. These challenges do not only affect people with CF (pwCF) in Africa but also have implications for global understanding of the disease, particularly among populations historically excluded from CF research and treatment advances.
View Article and Find Full Text PDFLancet Respir Med
September 2025
Department for Paediatric Pneumology, Allergology, and Neonatology and German Center for Lung Research, Biomedical Research in Endstage and Obstructive Lung Disease, Hannover Medical School, 30625 Hannover, Germany. Electronic address:
Lancet Respir Med
September 2025
Effi-Stat, Paris, France.
Background: Among people with cystic fibrosis, sweat chloride and lung function response to elexacaftor-tezacaftor-ivacaftor (ETI) is variable. We hypothesised that the presence of two versus one ETI-responsive CFTR variant could predict response variability.
Methods: In this analysis of two real-world observational studies, data from a French national cohort of adults (aged ≥18 years) with cystic fibrosis and at least one F508del variant treated with ETI and the French compassionate programme for ETI in people (aged ≥6 years) with cystic fibrosis without F508del were used to examine sweat chloride concentrations (SCCs) after ETI initiation, and the absolute change in SCC and percentage of predicted forced expiratory volume in 1 s (ppFEV) following ETI initiation.
Int J Surg Case Rep
September 2025
Department of Internal Medicine, Pulmonology and Critical Care Unit, Bahir Dar University, Bahir Dar, Ethiopia.
Introduction And Importance: Lymphangioleiomyomatosis (LAM) is a rare disorder of unknown cause which mostly affects young females and involving multi organ system with primarily involving lung.
Presentation Of Case: A 35 year's old female Ethiopian known hypertension patient from Debre Tabor, Ethiopia, Africa; presented with progressively increasing cough with blood tingled sputum of 1-2 Arabic coffee cup per day, progressively increasing exertional shortness of breath and easy fatigability seven years back. Hypertensive and desaturate to level of 88 % at atmospheric air.
Thorax
September 2025
Clinical Trials Accelerator Platform, London, UK
A common eligibility criterion in respiratory clinical trials is a per cent-predicted forced expiratory volume in 1 second (ppFEV) between 40% and 90%, using the ethnicity-dependent Global Lung Function Initiative (GLI)-2012 spirometry reference equations. International societies now endorse the newer 'race-neutral' GLI-Global equations. We quantify the impact on trial eligibility of switching from GLI-2012 to GLI-Global for the UK Cystic Fibrosis Registry (n=8182).
View Article and Find Full Text PDF